Undifferentiated high-grade pleomorphic sarcoma (UHPS) is a highly aggressive sarcoma that presents a considerable degree of cellular pleomorphism and undifferentiation. It was formerly called malignant fibrous histiocytoma of bone. Recently it has been diagnosed by immunohistochemical (IHC) staining. To the best of our knowledge, only 19 cases have been reported in the literature. Of these, IHC staining was examined in only 13 cases. This paper reports a case of UHPS of the mandible with some literature review.
The patient was a 62-year-old woman with a 52 × 27 mm mass on the right mandibular body with bone
destruction.
Biopsy specimens revealed osteosarcoma. We performed a right hemimandibulectomy and ipsilateral neck dissection. It took five months to diagnose UHPS with IHC staining. After the surgery, lung metastasis was observed and lung surgery was performed. One year after the lung surgery, local recurrence was observed and heavy ion radiotherapy was carried out. The treatment result was CR. However, the lung metastasis advanced rapidly, and lung surgery and chemotherapy were performed. Despite this, her general condition gradually deteriorated and she died 1.5 years after diagnosis.
